Guillain-Barré syndrome (GBS) is a rare disorder of the peripheral nervous system. The syndrome is named after the French physicians Georges Guillain and Jean Alexandre Barré, who described it in 1916. It is also called acute inflammatory demyelinating polyneuropathy (AIDP).
GBS is an autoimmune disorder in which a person’s own immune system attacks healthy nerve cells in peripheral nervous system. The syndrome can affect the nerves that control muscle movement and nerves that transmit feelings of pain, temperature and touch. This leads to muscle weakness, numbness, and tingling and sometimes paralysis.
Most people recover fully from GBS, but some people have long-term nerve damage. 3%-5% of GBS patients may die from complications, which include paralysis of the muscles that control breathing, blood infection, lung clots or cardiac arrest.
The cause of this condition is unknown, but it’s typically triggered by an infectious illness, such as the gastrointestinal infection or a lung infection. Few countries from Europe and Asia have reported familial occurrence of GBS (42 GBS patients found from 20 families). In a hospital based study from South India among 150 GBS cases reported during 10 years period, 2 cases belonged to a single familya.
According to WHO overall incidence of GBS is 0.4 to 4.0 people per 100 000 per year. People of all ages can be affected, but it is more common in adults and in males.
During 2015 and 2016, nine countries and territories(El Salvador, French Polynesia, Martinique, Colombia, Suriname, Puerto Rico, Brazil, Panama, Venezuela (Bolivarian Republic of) have reported an increased incidence of Guillain-Barré syndrome (GBS) and/or laboratory confirmation of a Zika virus infection among GBS casesb. Researchers are studying a potential link between the surge in GBS cases and Zika virus infection. Zika virus is not yet proven to be the cause of the increased GBS incidence in Brazil, Colombia, El Salvador, Suriname or Venezuela (Bolivarian Republic of).
References-
who.int/mediacentre/factsheets/
healthline.com/health/guillain-barre
gbs-cidp.org/wp-content/uploads/
who.int/csr/don/12-february-2016-
a Aik KR et al, Familial Guillain-Barré syndrome: First Indian report. Ann Indian Acad Neurol [serial online] 2012[cited 2016 Mar 10]:15:44-7.available from .annalsofian.org/text.asp
Symptoms usually start with numbness or tingling in the fingers and toes. Over several days, muscle weakness develops in the legs and arms. The lower limbs are usually involved before the upper limbs. Weakness is ascending and symmetrical in nature. The weakness may progress to the arms, face (In 45%-75% of patients III-VII and IX-XII cranial nerves are involved), and muscles of respiration (the chest muscles are affected in 20%-25% of cases).
The symptoms of Guillain-Barré syndrome include:
Most people recover fully from the disease; however some cases continue to experience weakness.
References-
who.int/mediacentre/factsheets
The etiology of GBS is not completely understood. It is not a contagious disorder.
About two-thirds of people with GBS develop it soon after they have been suffered with diarrhea or a respiratory infection. This suggests that the disorder may be triggered by an improper immune response to the previous illness.
Infections that may trigger GBS include:
GBS can affect person of any age group, however the incidence of GBS increases with age. People older than 50 years are at greatest risk for developing GBS.
References-
webmd.com/brain/tc/guillain-barre-syndrome
cdc.gov/flu/protect/vaccine/guillainbarre.htm
healthline.com/health/guillain-barre-syndrome
Guillain-Barré syndrome is difficult to diagnose because the symptoms are similar to other neurological disorders. Diagnosis is based on symptoms, findings on neurological examination including diminished or loss of deep-tendon reflexes.
Routine blood tests are done, to exclude other diseases with similar symptoms and for better assessment of functional status and prognosis.
Specific tests are required to identify the cause of trigger of GBS.
The following tests are used to confirm a diagnosis of GBS-
On 25th February 2016, under Identification and management of Guillain-Barré syndrome in the context of Zika virus, WHO has recommended using Brighton criteria as the case definition of GBS, so that standardized information may be available for epidemiologic purposes(not as a criterion for treatment). This criterion is based on presenting clinical findings and ancillary testing including neurophysiology and lumbar puncture findings. Patients are categorized as level-1 (the highest level of diagnostic certainty) to level -3(the lowest level of diagnostic certainty)*.
References-
emedicine.medscape.com/article/
healthline.com/health/guillain-barre-syndrome
*Identification and management of Guillain-Barré syndrome in the context of Zika virus, available from-
The goal of treatment is to lessen the severity of symptoms and maintain vital functions normal while nervous system recovers.
(a)Supportive therapy-
(b) Immunotheraphy: Immunotherapy comprises IVIG or plasma exchange.
(c) Rehabilitation services:
References-
who.int/mediacentre/factsheets
Most people recover fully from GBS, but some people have long-term nerve damage. Residual weakness can be seen in 30% of GBS cases after three years. About three percent of cases may suffer a relapse of muscle weakness and tingling sensations many years after the initial attack. 3%-5% of GBS patients may die from complications, which include;
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Primary prevention:
Guillain-Barré syndrome is a condition (not a disease itself), and its causation is not known exactly. Therefore no specific preventive measure can be indicated.
Sometimes vaccination may trigger the occurrence of GBS; hence vaccination is not suggested in acute phase and up to a period of one year after an episode of GBS.
As there is increase in cases of Guillain-Barré syndrome in areas where Zika virus is circulating and researchers are investigating to prove the link between the two, WHO recommends that anyone living in or traveling to areas where the Zika virus is circulating take precautions to avoid mosquito bites. For additional information on prevention of Zika virus infection, click here (http://www.nhp.gov.in/disease/communicable-disease/zika-virus-disease)
Secondary prevention:
When possible, patients should be treated in an intensive care unit in order to keep continuous monitoring and to respond immediately to any urgency. Thus complications due to disease and immobility can be identified and responded early by healthcare workers.
Patients of Guillain-Barré syndrome suffer not only physical difficulties but emotionally painful periods also. Residual symptoms may lead to long term disability and cause difficulties in attaining prior lifestyle or occupation. Rehabilitation services combined with psychological counseling would be helpful in recovery of the patients.
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Variants of GBS are-
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